Pediatric emergency medicine trisk 2073 2073
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The incidence of BA varies based on geography, occurring most commonly in
East Asia with an incidence of 1 in 5,000 births in Taiwan and 1 in 10,000 to
15,000 births in the United States. It is the leading cause of liver transplant
and death from liver failure in children. Girls are slightly more affected than
boys, and it occurs in all races and ethnicities. Data suggest that patients with
BA have an elevated conjugated bilirubinemia shortly after birth, as early as
within the first 24 to 48 hours. There is no universal screening tool at present,
although some countries have had success with stool color cards as a public
health measure to help families identify acholic stools ( Fig. 91.2 ). The cause
of BA remains unknown. There are several subtypes of BA, but the end result
in all patients is that there is an inflammatory process that destroys both
intrahepatic and extrahepatic biliary tracts and leads to complete biliary
obstruction, liver damage, progressive liver cirrhosis, and liver failure.
Treatment is surgical, which restores biliary flow via a
hepatoportoenterostomy. There is strong evidence that early diagnosis and
surgical correction of BA improves both long-term morbidity and mortality.
Patients who received surgical correction prior to 60 days of life had a 10-year
survival rate of 73%, whereas patients who received surgery after 90 days of
life had a 10-year survival rate of 11%. Unfortunately, the mean age for
surgical correction in the United States is 65.5 days of life, which has not
improved in the past 20 years. Up to 20% of patients have other congenital
abnormalities. The most common co-occurring congenital abnormalities are
splenic malformations (polysplenia), situs inversus, and other vascular
abnormalities. Patients are also at risk for congenital cardiac defects and
intestinal malrotation.
Clinical Considerations
Clinical Recognition. Initially, patients with BA are normal in appearance, but
do have an elevated conjugated hyperbilirubinemia. Patients subsequently
have persistent jaundice and develop acholic stool. Without intervention, all
patients will develop end-stage liver disease by 1 year of age. A clinician must
have a high index of suspicion for patients with persistent jaundice. There are
many causes of jaundice in a newborn (see Chapters 44 Jaundice: Conjugated
Hyperbilirubinemia and 45 Jaundice: Unconjugated Hyperbilirubinemia ). In
general, 15% of newborns are jaundiced at 2 weeks of age and 2% to 6% at 4
